ABSTRACT
Idiopathic steroid-resistant nephrotic syndrome [ISRNS] is rare and represents a significant therapeutic dilemma for paediatricians and paediatric nephrologists.To analyze characteristics of the ISRNS in the child. Retrospective study of 20 cases of ISRNS enrolled in paediatric department of nephrology in Sahloul hospital [Tunisia] between June 1993 and December 2007 [14 years period]. There were eight girls and 12 boys [mean age: 5.8 +/- 3.7 years] originating from the center or the south of Tunisia. Eight of them had a minimal-change disease [MCD], 11 a focal and segmental glomerulosclerosis [FSGS] and one a mesangioproliferative glomerulonephritis [MePGN]. In this group, no family form could be identified. All patients were treated by cyclosporine associated with low dose of steroid. We noted a complete remission [CR] in nine cases, partial remission [PR] in three cases and no response to cyclosporine in eight cases. Among patients with CR, six presented MCD and three a FSGS. In this group, we observed relapse of nephrotic syndrome in six cases. End stage renal disease [ESRD] was noted in 10 patients of which five not responded to cyclosporine, two initially having presented a RC and three having since the beginning a PR. Among them, two only could be grafted; one relapses on transplant was observed with a single patient initially presenting a secondarily transformed MePGN in FSGS. Our study confirms the clinical, histological and evolutive heterogeneity of idiopathic steroid-resistant nephrotic syndrome. Although there is any therapeutic consensus in this domain, cyclosporine remains indicated in first intention in sporadic forms of ISRNS. On the other hand, renal transplantation constitutes the only therapeutic alternate in genetic forms that constantly evolve at ESRD
ABSTRACT
Renal involvement is one of the most severe and frequent manifestations of the systemic lupus erythematosus [SLE]. In this study, we analyzed clinical and evolutive particularities of 14 paediatric cases of lupus nephritis [LN]. It's a retrospective study in 14 children with lupus nephritis followed-up in the paediatrics department of Sousse and Mahdia between 1983 and 2004. There were 12 girls and two boys [sex - ratio = 0.16] aged four to 14 years [mean age =10 years]. At the first presentation, we noted proteinuria in all patients with nephrotic syndrome in six cases, hypertension with variable severity in five cases, hematuria in six cases and a variable severity of renal insufficiency in six cases. Histological examination of kidney performed in 10 patients with severe nephropathy, revealed class IV glomerulonephritis in four cases, class V in two cases and class III in four cases. Thirteen patients were treated by corticosteroids associated with immunosuppressive agent in six cases. One patient had not received any treatment. Five patients were dead of the continuations of SLE complications or immunosuppressive therapy. For the other patients, one is in clinical and biological remission since six years, four are lost of view, one is in end stage renal failure, two presented relapsing evolution and one presents refractory form of LN. Lupus nephritis is severe in our patients with predominance of class III and IV. New therapeutic strategies permitted to improve the renal survival but at the cost of an important iatrogenic morbidity